We experienced a case of living renal transplantation from a horseshoe kidney donor. The recipient was a 32-year-old man with end-stage renal failure. At 20 years old, he received a living renal transplantation from his mother. However, he received dialysis again for chronic rejection at the age of 31. The donor was his healthy 66-year-old father, who had a horseshoe kidney. Dynamic computed tomography (d-CT) showed two renal arteries and one renal vein on the left side, and the isthmus was supplied by several accessory arteries and veins. The donor surgery was performed through an abdominal midline incision. The accessory arteries were clamped to demarcate the boundary of the isthmus. After the isthmus parenchyma was clamped, the kidney was divided at the boundary by electric scalpel. Both cut ends of the isthmus were closed by sutures. The left kidney was transplanted into the left iliac fossa of the recipient. The donor was discharged on the 9th day after surgery without perioperative complications. Likewise, the recipient experienced no surgical complication or rejection. Kidney functions of donor and recipient have been stable for a 12-month follow-up period. We believe horseshoe kidneys may be used for living donor transplantation in selected cases after a detailed preoperative evaluation.